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Fulminant JC virus encephalopathy with productive infection of cortical pyramidal neurons†

  1. Christian Wüthrich PhD1,
  2. Xin Dang PhD1,
  3. Susan Westmoreland VMD2,
  4. John McKay BSc2,
  5. Atul Maheshwari MD3,
  6. Matthew P. Anderson MD, PhD4,5,
  7. Allan H. Ropper MD3,
  8. Raphael P. Viscidi MD6,
  9. Igor J. Koralnik MD1,2,3,4,*

Article first published online: 18 MAR 2009

DOI: 10.1002/ana.21619

Issue

Annals of Neurology

Annals of Neurology

Volume 65, Issue 6, pages 742–748, June 2009

Additional Information

How to Cite

Wüthrich, C., Dang, X., Westmoreland, S., McKay, J., Maheshwari, A., Anderson, M. P., Ropper, A. H., Viscidi, R. P. and Koralnik, I. J. (2009), Fulminant JC virus encephalopathy with productive infection of cortical pyramidal neurons. Annals of Neurology, 65: 742–748. doi: 10.1002/ana.21619

Author Information

  1. 1

    Division of Viral Pathogenesis, Beth Israel Deaconess Medical Center, Beth Israel Deaconess Medical Center, Boston, MA

  2. 2

    Department of Comparative Pathology, New England Primate Research Center, Beth Israel Deaconess Medical Center, Boston, MA

  3. 3

    Department of Neurology, Brigham and Women's Hospital, Beth Israel Deaconess Medical Center, Boston, MA

  4. 4

    Department of Neurology, Beth Israel Deaconess Medical Center, Boston, MA

  5. 5

    Department of Pathology, Beth Israel Deaconess Medical Center, Boston, MA

  6. 6

    Department of Pediatrics, Johns Hopkins University School of Medicine, Baltimore, MD

*BIDMC, 330 Brookline Avenue, E/CLS A005, Boston, MA 02215

  1. †

    Potential conflict of interest: Nothing to report.

Publication History

  1. Issue published online: 25 JUN 2009
  2. Article first published online: 18 MAR 2009
  3. Accepted manuscript online: 18 MAR 2009 12:00AM EST
  4. Manuscript Accepted: 21 NOV 2008
  5. Manuscript Revised: 29 OCT 2008
  6. Manuscript Received: 26 AUG 2008

Funded by

  • Public Health Service. Grant Numbers: R01 NS 047029, R01 NS 041198, K24 NS 060950, R01 NS 057444, 1K02NS054674

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Abstract

The polyomavirus JC (JCV) is the causative agent of progressive multifocal leukoencephalopathy and of JCV granule cell neuronopathy. We present a human immunodeficiency virus–negative patient who experienced development of multiple cortical lesions, aphasia, and progressive cognitive decline after chemotherapy for non–small-cell lung cancer. Brain biopsy and cerebrospinal fluid polymerase chain reaction demonstrated JCV, and she had a rapidly fatal outcome. Postmortem analysis showed diffuse cortical lesions and areas of necrosis at the gray–white junction. Immunostaining showed a productive JCV infection of cortical pyramidal neurons, confirmed by electron microscopy, with limited demyelination. This novel gray matter syndrome expands the scope of JCV clinical presentation and pathogenesis. Ann Neurol 2009;65:742–748

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